Fasciculation cramp syndrome preceding anterior horn cell disease
:an intermediate syndrome?
While the clinical-electrophysiological profile was suggestive of anterior horn cell disease
, dynamic flexion MRI of the cervical spine supports the theory of forward dural shift causing lower cervical cord compression and ischaemia of anterior horns.
While the presence of reflexes is reassuring, absence suggests a peripheral nerve problem or anterior horn cell disease
Although muscle denervation and reduced CMAP amplitudes do not distinguish loss of anterior horn cells from loss of motor axons (15), these patients' clinical features can be explained only by anterior horn cell disease
, since no known infectious processes limited to motor axons produce widespread, asymmetric paralysis without sensory involvement.
* Progressive muscle weakness, most severely affecting the legs and leading to paralysis, and identical to the pattern of weakness seen in children with spinal muscular atrophy (anterior horn cell disease
We report a 14-year old boy with an unusual presentation of extensive bilateral macular coloboma with planovalgus feet dynamics and left foot drop due to anterior horn cell disease